What are the other Names for this Condition? (Also known as/Synonyms)

• Dabska Tumor
• Endovascular Papillary Angioendothelioma of Childhood
• Malignant Endovascular Papillary Angioendothelioma

What is Papillary Intralymphatic Angioendothelioma? (Definition/Background Information)

• Papillary Intralymphatic Angioendothelioma (PILA) is an infrequent, aggressive tumor, involving blood vessels and lymphatic-like channels. They are regarded as a rare type of lymphangiosarcoma and they have malignancy potential (capacity to metastasize)
• PILA manifests itself during early childhood and these tumors are normally located on the skin surface, or lie beneath the skin tissues
• It is commonly found on the hands and legs, with other reported sites that include the head, neck, and torso
• Any combination of steroids, chemotherapy, radiation therapy, and invasive procedures are used to treat PILAs. However, the prognosis depends on many factors; the most important factor being the stage of the tumor, at the time of diagnosis

Who gets Papillary Intralymphatic Angioendothelioma? (Age and Sex Distribution)

• Papillary Intralymphatic Angioendothelioma is mostly observed during early infancy and childhood phase. However, 25% of the cases reported, are in adults
• PILA is seen in both the male and female sexes uniformly
• There is no known ethnic or racial preference

What are the Risk Factors for Papillary Intralymphatic Angioendothelioma? (Predisposing Factors)

A few risk factors for Papillary Intralymphatic Angioendothelioma include:

• PILA seems to be linked to a previously performed radiotherapy - it may be radiation-induced
• It is also thought to be sometimes associated with the chronic obstruction of the lymph vessels (termed as lymphedema)
• The presence of other skin cancers and vascular (blood vessel related) tumors are known to be connected to PILA

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Papillary Intralymphatic Angioendothelioma? (Etiology)

• The exact cause of Papillary Intralymphatic Angioendothelioma is unknown. They are thought to occur spontaneously
• It is suggested that PILA origin might be related to abnormal blood vessel proliferations, for unknown reasons

What are the Signs and Symptoms of Papillary Intralymphatic Angioendothelioma?

The signs and symptoms are based on the location of Papillary Intralymphatic Angioendotheliomas, and many of these tumors are asymptomatic. The signs and symptoms of PILA may include:

• They may grow at a slow rate and appear as painless inflammatory irregular or nodular lesions below the skin surface. Deep-seated tumors are generally infrequent
• Superficial PILAs might look like birthmarks - as red-blue or violet colored scars
• These tumors usually occur in the limbs. Some case reports have indicated that they may occur in the trunk, head and neck region, spleen, and tongue

How is Papillary Intralymphatic Angioendothelioma Diagnosed?

A diagnosis of Papillary Intralymphatic Angioendothelioma would involve:

• Physical examination with evaluation of patient’s medical history
• Histopathological studies conducted on a biopsy specimen: The biopsy is examined under a microscope by the pathologist, to arrive at a definitive diagnosis. The pathologist may also perform special studies, to assist in the diagnosis
• Fine needle aspiration of the tumor may also be performed, to procure specimen samples for diagnosis
• Imaging studies through CT scan and MRI scan of the affected region, which may also aid in surgery

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Papillary Intralymphatic Angioendothelioma?

Papillary Intralymphatic Angioendothelioma complications may include:

• They are known to recur after surgical excision
• Metastasis to different parts of the body including the regional lymph nodes and lungs. In a majority of the cases, the tumor spreads to the surrounding tissues, such as the muscles and bones (local spread of the tumor)
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery
• There may be other complications, if any vascular disorders, like lymphedema, are present
• Side effects from chemotherapy (such as toxicity), radiation therapy (such as radiation fibrosis)

How is Papillary Intralymphatic Angioendothelioma Treated?

• Wide surgical excision of PILA, with removal of the entire lesion is the standard treatment mode. If it is not fully removed, then there are chances of a recurrence
• If the tumor has metastasized to the regional lymph nodes, then removal of the lymph nodes (called lymph node dissection) may also be necessary
• Any combination of steroids, chemotherapy, radiation therapy, and invasive procedures are used to treat PILAs
• Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• When PILA is at an inaccessible location, or is unsafe for surgical intervention, non-invasive procedures are adopted
• Interferon injections are used to reduce tumor blood supply, and limit its growth
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Papillary Intralymphatic Angioendothelioma be Prevented?

• Current medical research have not established a way of preventing Papillary Intralymphatic Angioendothelioma
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its metastasizing potential and possibility of recurrence

What is the Prognosis of Papillary Intralymphatic Angioendothelioma? (Outcomes/Resolutions)

• Papillary Intralymphatic Angioendotheliomas are rare and not yet completely understood; the long-term prognosis depends on a combination of factors such as:
  • First appearance or detection of the tumor
  • Tumor size and location
  • The overall prognosis depends on the stage of the tumor
  • Its response to treatment and medical therapy
• Surface and sub-surface tumors that are well-defined can be completely excised, with low chances of recurrence. However, PILA has a latent potential to be fatal

Additional and Relevant Useful Information for Papillary Intralymphatic Angioendothelioma:

• PILA is said to be closely related to Retiform Hemangioendothelioma (a highly infrequen,t low-grade angiosarcoma)
• The pathologist may have to consider other differential diagnosis, prior to a definite diagnosis of Papillary Intralymphatic Angioendothelioma. The tumors to consider in the differential diagnosis may include:
  • Infantile Hemangioma
  • Acquired Digital Fibrokeratoma
  • Kaposi Sarcoma
  • Acute Hemorrhagic Edema of Infancy
  • Angiolymphoid Hyperplasia with Eosinophilia